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Objective The skin pathergy test (SPT) is an important tool in the diagnosis of Behçet disease (BD), but its decreasing sensitivity over years has resulted in less frequent use in the clinical practice. This study aimed to improve the sensitivity of the SPT without compromising its specificity. Methods BD patients, patients with other inflammatory diseases, recurrent aphthous stomatitis, and healthy controls comprised the study group. The SPT was conducted using 20G needle and 21G lancet pricks, or with additional application of 23-valent polysaccharide pneumococcal vaccine (PS-23), Alum, or ATP to the prick site. Development of erythema and induration at 24 h/48 h were evaluated by the same observer. Induration (≥2 mm) with erythema at 48 h was accepted as a positive reaction. Proinflammatory cytokine production following stimulation with lipopolysaccharide or PS-23 was investigated by whole-blood assay (WBA) in a subgroup. Results Stimulation of the forearm skin by PS-23 and a 20G needle prick showed the highest sensitivity and specificity in BD (64.3% and 100%, respectively), especially in patients with active disease (80.3% and 100%, respectively), compared with a sensitivity of 4.8% in all and 6.1% in active patients using a single 20G prick. A positive result was associated with active disease and no use of immunosuppressives. In WBA, increased IL-1β and IL-1Ra production in response to PS-23 was observed in the group with active BD, while the cytokine response to lipopolysaccharide was similar in all groups. Conclusions The SPT conducted using a 20G needle prick and PS-23 antigens was shown to be a promising tool for the diagnosis of BD owing to its improved sensitivity compared with the standard approach. [ABSTRACT FROM AUTHOR]

The HLA protein, HLA-B*51, encoded by HLA-B in MHC, is the strongest known genetic risk factor for Behçet disease (BD). Associations between BD and other factors within the MHC have been reported also, although strong regional linkage disequilibrium complicates their confident disentanglement from HLA-B*51. In the current study, we examined a combination of directly obtained arid imputed MHC-region SNPs, directly obtained HLA-B locus types, and imputed classical HLA types with their corresponding polymorphic amino acid residues for association with BD in 1,190 cases and 1,257 controls. SNP mapping with logistic regression of the MHC identified the HLA-B/MICA region and the region between HLA-F and HLA-A as independently associated with BD (P < 1.7 × 10-8). HLA-B*51, -A*03, -B*15, -B*27, -B*49, -B*57, and -A*26 each contributed independently to BD risk. We directly examined rsi 16799036, a noncoding SNP upstream of HLA-B that was recently suggested to underlie the association of HLA-B*51 with BD, but we were unable to replicate that finding in our collection. Instead, we mapped the BD association to seven MHC class I (MHC-I) amino acid residues, including anchor residues that critically define the selection and binding of peptides to MHC-I molecules, residues known to influence MHC-Ikiller immunoglobulin-like receptor interactions, and a residue located in the signal peptide of HLA-B. The locations of these variants collectively implicate MHC-I peptide binding in the pathophysiology of BD. Furthermore, several lines of evidence suggest a role for altered regulation of cellular cytotoxicity in BD pathogenesis. [ABSTRACT FROM AUTHOR]

Objectives. There had been evidence that low-dose local IFN could be beneficial in the management of recurrent oral ulcers (OUs). We investigated the efficacy and collected initial data on the safety of low-dose natural human IFN-α administered by the oral mucosal route in Behçet's syndrome (BS) in a placebo controlled, double blind study. Methods. Eighty-four (59 males and 25 females) patients with BS with mainly skin mucosa disease and a history of recurrent OU for ≥ 1 year were studied. When they had at least two OUs with a total diameter of ≥ 4mm, they were randomly allocated to (i) 2000 IFN-α IU/day, (ii) 1000 IFN-α IU/day and (iii) placebo groups. Subjects were monitored weekly over an initial 4 weeks and bi-weekly for an additional 8 weeks of treatment. OU were counted and measured at each study visit. The primary efficacy end point was the difference in the total ulcer burden at Week 0 compared with that at Week 12. Results. Out of the 84 patients enrolled, 72 completed the trial. There were no statistically significant differences between the treatment arms in terms of the primary endpoint. Conclusions. Low-dose natural human IFN-α did not have beneficial effects on reducing the total ulcer burden among BS patients from Turkey. The study also showed that counting the number of ulcers rather than measuring the size would be adequate in future studies. Trial registration. ClinicalTrials.gov, NCT00483184, http://www.clinicaltrials.gov/ct2/results?term=NCT00483184 [ABSTRACT FROM AUTHOR]